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PubMed2000

Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia. Case report.

The spontaneous resolution of syringomyelia has been reported infrequently. In patients with Chiari I malformations, resolution of the syringomyelia has sometimes been associated with improvement of their malformation. The authors present a case of spontaneous resolution followed by recurrence of syringomyelia and a corresponding change in the Chiari malformation. This case is of interest in light of the theories postulated to explain spontaneous resolution of syringomyelia.

Arnold-Chiari Malformation↗
PubMed2002

Evaluation of CSF motion in syringomyelia with spatial modulation of magnetization (SPAMM).

CSF flow dynamics has been known to have a key role the pathogenesis of syringomyelia. Our purpose was to evaluate the CSF flow pattern in syringomyelia with spatial modulation of magnetization (SPAMM) that can depict directional flow, and to determine the relationship between flow dynamics and clinical outcome after decompress surgery. We performed pre- and post-operative MRI in 9 patients with syringomyelia with SPAMM technique. They showed caudal shift of tagging bands in the syrinx cavity in systolic phase while stagnation of CSF flow was seen in diastolic phase. The degree of band shift in syrinx was well correlated with the degree of shrinkage in size of syrinx after operation. Evaluation of syringomyelia by SPAMM technique can predict postsurgical outcome after decompress surgery.

Adult↗
PubMed1999

[Neuroembryologic considerations on the so-called malformative syringomyelia].

Modern neuroradiological techniques can evidence the presence of liquid-filled spaces within the spinal cord, called syringomyelia. These lesions may be associated with numerous causes, the most frequent of which is an abnormality of the shape of the posterior fossa. Neuropathological analysis of these cavities demonstrates whether they are completely lined by ependymal cells or not. Comparing neuropathological and embryological data suggests that syringomyelia is a secondary deformation affecting a normally-formed spinal cord. The unique case in which such a cavity is really a primary malformation is the so-called myelocytocele. The most frequently encountered lesion associated with syringomyelia is the Chiari abnormality (either type I or II). In this case, the size of the posterior fossa is too small whereas neural elements are normal. Since Chiari abnormality may be familial, some genes are likely to be involved for its generation. In experimental animals, it has been shown that genes belonging to the Hox family or the Mhox gene control the development of the final shape of the occipital bone. Syringomyelia is thus a secondary event affecting the spinal cord and due to a distant cause.

Humans↗
PubMed1999

[Imaging of syringomyelia].

MRI is the best imaging method to evaluate syringomyelia. It is important to study from the posterior cranial fossa to the sacro-lumbar region and also the supra-tentorial structures. This complete analysis is essential to classify the syringomyelia and to investigate other associated malformations. Radiographs and CT scan are useful to analyze bone structures. For MRI, the new sequences with phased-array coils are also very important to study the entire spinal cord and the posterior fossa. It is essential to study the spinal cord with sagittal and axial spin echo T1 and fast spin echo T2 weighted images with sometimes coronal view, particularly when the patient presents a scoliosis, to have a correct morphological and functional evaluation. MRI gives an excellent study of the spinal cord with an excellent analysis of a primitive or foraminal syringomyelia, but also traumatic, infectious or post arachnoiditis syringomyelia. Spin echo T1 weighted images with injection of gadolinium can be used if an intra-medullary tumor is suspected. MRI is also useful for the post-operative follow up to evaluate the persistence of the medullary cyst and the enlargement of the foramen magnum.

Brain↗
PubMed1999

Electrodiagnostic findings in syringomyelia.

Electrodiagnostic abnormalities are well known to occur in syringomyelia although the findings are nonspecific. The objective of this work was to describe different types of spontaneous electromyographic (EMG) activity and reflex responses, which may be useful and more specific than conventional findings for the electrodiagnosis of syringomyelia. We studied 43 patients with syringomyelia by four-channel surface EMG and by recording the long-latency responses to distal stimulation of the median and tibial nerves. Continuous motor unit activity (CMUA) was found in 18 patients, synchronous motor unit potentials (SMUP) in 10, respiratory synkinesis (RS) in 5, and myokymic discharges in 4. Long-latency responses (LLR) with latencies ranging from 55 to 150 ms were found in 14 patients. Patients with syringomyelia thus show a wide variation of spontaneous EMG activity. An increase in excitability of spinal motor neurons is probably the basic underlying mechanism.

Adolescent↗
PubMed2000

Retrocerebellar arachnoid cyst with syringomyelia: a case report.

Association of syringomyelia with retrocerebellar arachnoid cysts is rare. A case of 14 year old female is being reported, who presented with hydrocephalus caused by a large midline retrocerebellar infravermal arachnoid cyst leading to obstruction of the outlet foramina of the fourth ventricle. There was associated syringomyelia. The pathogenesis of syringomyelia is discussed. The need to evaluate cervical spinal cord by taking T1 weighted sagittal sections in all the patients of large posterior fossa mass lesions causing obstruction to the outlet foramina of the fourth ventricle has been stressed, in order to detect associated syringomyelia.

Adolescent↗
PubMedDate not supplied

[Syringomyelia associated with intracranial tumors. Case report and literature review].

A case of 39 years old woman with two intracranial meningiomas and syringomyelia is presented. Large right-sided tentorial meningioma in cerebellopontine angle and middle cranial fossa and small left sided sphenoid wing meningioma co-existed with secondary tonsillar herniation and large syringomyelic cavity in cervical and thoracic spinal cord. The patient had dissociated sensory loss on trunk and upper left extremity, muscle atrophy, left hand paresis, long tracts signs. After having done atlanto-occipital decompression, the intramedullary cavity collapsed and neurological symptoms resolved. Two months later large tentorial meningioma was successfully removed via occipito-suboccipital craniotomy with tentorial transsection. A review of the literature concerning syringomyelia secondary to intracranial tumours was done. To our knowledge this is the first such case described in which syringomyelia syndrome was the prominent symptom of the disease and two staged surgical procedure, first oriented at treatment of syringomyelia itself, was applied.

Adult↗
PubMed2001

Localized hypertrophy of hand in syringomyelia.

Segmental amyotrophy is a common feature resulting from extension of the syrinx into the anterior horns. Segmental hypertrophy in the form of chiromegaly/podomegaly or limb and hemi-hypertrophy are rare but well recognized clinical feature of syringomyelia. We report a case of hand hypertrophy secondary to syringomyelia. Hypertrophy of the hands is a rare but interesting feature of syringomyelia and in all cases of enlargements of hands syringomyelia should be considered in the differential diagnosis.

Adult↗
PubMed2002

Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations.

OBJECTIVE AND IMPORTANCE: A Chiari I malformation associated with syringomyelia and hydrocephalus is a rare condition. We report the successful use of endoscopic third ventriculostomy for the treatment of this pathological entity. The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved. CLINICAL PRESENTATION: A 34-year-old woman presented with headaches, a motor deficit of the right upper limb, and gait dyspraxia. Magnetic resonance imaging scans demonstrated dilation of all ventricles, compression of the retrocerebellar cerebrospinal fluid space, downward displacement of the tonsils, and syringomyelia. Syringomyelia involved the cervicodorsal cord below C3, with a syrinx-free segment between C1 and C3 and no enlargement of the rostral part of the central canal. INTERVENTION: Endoscopic third ventriculostomy resulted in prompt improvement of the clinical symptoms. Postoperative magnetic resonance imaging scans demonstrated shrinkage of the syrinx and return of the cerebellar tonsils to their physiological positions. CONCLUSION: This experience demonstrates that endoscopic third ventriculostomy, which is a simple, safe technique, may be the treatment of choice for associated Chiari I malformations, hydrocephalus, and syringomyelia (even the noncommunicating type).

Adult↗
PubMed1977

[Prevalence and features of the course of syringomyelia in the Samarkand region of the tuzbek SSR].

The paper is concerned with a clinico-statistical study of syringomyelia according to the data of neurological hospitals in the Samarkand region from 1931--1970. It was established that the portion of syringomyelia among other diseases of the nervous system is much lower (0.38%) than in other more northern regions of the country (3--7%). The author marks a significantly greater amount of patients among the city population rather than in the rural population (2/3) and a prevalence of non-natives over natives (more than 2/3). The development of syringomyelia is more milder, "benign", especially in the native population. Quite possibly this may be connected with the climatic-geographical and bio-geochemical traits of the Zarafshanskaya valley, which differs to a great extent from these indices of the territory between the Vyatka and Kama rivers where syringomyelia is frequently encountered.

Adult↗
PubMed2004

Clinical manifestations and significance of post-traumatic thoracolumbar syringomyelia.

OBJECTIVE: To analyze the pathogenic mechanism and the clinical significance of post-traumatic thoracolumbar syringomyelia through reviewing the clinical manifestations. METHODS: The data of 15 patients (14 males and 1 female, aged from 28 to 56 years, with an average of 36 years) with post-traumatic syringomyelia treated in our hospital from December 1997 to February 2002 were studied retrospectively. Two patients suffered from T11 fractures, 7 from T12 fractures and 6 from L1 fractures. There were 12 patients with burst fractures and 3 with fracture dislocations. Anterior decompression, bone graft, bone fusion and internal fixation were made on 6 patients, posterior decompression, bone graft, bone fusion and internal fixation on 1 patient, and non-surgical treatment on 8 patients. RESULTS: Syringomyelia of the patients was diagnosed accurately with magnetic resonance imaging at 0.5-4 years after the original thoracolumbar fracture. The cavern was round in 6 cases, elliptic in 6 cases, and irregular in 3 cases. The patients also suffered from pain (80%), myodynamia attenuation in lower extremities (66.7%), aggravated spasm (46.7%), sensation loss or hypesthesia (46.7%), decreased coordinate function of lower extremities (20%) and autonomic nerve symptom (6.7%). CONCLUSIONS: Post-traumatic thoracolumbar syringomyelia should be suspected if the patient has new neurological symptoms, such as myodynamia attenuation in lower extremities, after the neural function becomes stable for certain time.

Adult↗
PubMed1992

[Quantitative analysis of cerebrospinal fluid dynamics in syringomyelia using cine MRI with pre-saturation].

Some reports show the qualitative analysis of cerebrospinal fluid (CSF) pulsation in the subarachnoid space and the syrinx using cine magnetic resonance imaging (MRI). However, few reports studied the quantitative analysis of CSF pulsation. We report here the results of quantitative analysis of CSF pulsation using the cine MRI with pre-saturation pulse. Using flow phantom, we calibrated the correlation between the true velocity and calculated velocity acquired from the movement of pre-saturated low signal. Three cases of syringomyelia with Chiari malformation, two cases of traumatic syringomyelia, and three normal volunteers were examined using this technique, and we could obtain time-velocity curves of CSF in both subarachnoid spaces and syrinx. Although obvious pulsation of CSF was observed in the syrinx of all Chiari malformations, no pulsation of CSF was observed in the syrinx of traumatic syringomyelia. CSF in the syrinx moves upward in the early systolic cardiac cycle, downward in the systolic cycle, and upward again in the diastolic cycle. Patterns of CSF pulsation in syrinx were similar to those in the subarachnoid space, but "phase" of these pulsations differed from case to case. These results suggest the close relationship between CSF pulsation in the syrinx and CSF obstruction at the cranio-vertebral junction. Dissociation of the "phase" of CSF pulsation in the syrinx and subarachnoid space may be significant in the analysis of the pathogenesis of syringomyelia.

Adult↗
PubMed1991

[A surgical treatment method in syringomyelia].

The author provides pathogenetic evidence for surgical treatment of syringomyelia. Based on the experience gained with surgical treatment of syringomyelia, gives a critical assessment of the hydrodynamic theory of the formation of intraspinal cavities. Provides the data obtained during examination of 127 patients suffering from genuine syringomyelia with the aid of cystography and NMR tomography. The communicating and non-communicating disease pattern were established. The short- and long-term results of surgical treatment were elucidated in 109 patients operated on. It is assumed that derangement of hydrodynamics plays a definite part in the disease pathogenesis. The hydrodynamic alterations include not only circulatory disorders but also have the signs of general functional insufficiency of the CSF system. The surgical treatment method is aimed at the correction of the hydrodynamic alterations, being one of the constituent parts in the whole complex of treatment measures for syringomyelia.

Adolescent↗
PubMedDate not supplied

Paraplegia, syringomyelia tarda and neuropathic arthrosis of the shoulder: a triad.

A casually related triad of syringomyelia tarda, postparaplegia with secondary neuropathic arthrosis of the shoulder has been presented. The development of neuropathic arthrosis of the shoulder has been presented. The development of neuropathic arthrosis of the shoulder in 2 of our paraplegic patients prompted us to look for a correlation and/or a common etiology. Paraplegia secondary to spinal cord injury could be causative or at least an associated factor in a delayed proximal syringomyelia. It is the syringomyelia which can lead to the neuropathic arthrosis in the upper extremity, mainly, in the shoulder. There is an interesting pathological association of paraplegia and syringomyelia with neuropathic arthrosis. Earlier awareness of any neurological changes in the upper extremity of the paraplegic patient could point to recognition of syringomyelic process and portend a tendency toward neuropathic arthrosis of the shoulder. This would require a high index of suspicion, and subsequently a preventative, protective orthopedic approach to minimize the usual functional deformity that occurs when this neuropathy involves the shoulder.

Adult↗
PubMed1989

[A case of syringomyelia associated with postoperative extradural cyst and adhesive arachnoiditis].

We report a rare case of syringomyelia associated with postoperative adhesive arachnoiditis and an extradural cyst. A 61-year-old male was admitted to our hospital five years after surgical removal of a cervical neurinoma. He complained of progressive quadriparesis and sensory disturbance. A CT scan showed a large cyst posteriorly within the C2, 3, 4 laminectomy area. MRI revealed syringomyelia with an extradural cyst at the C2, 3, 4 level. The operative findings revealed adhesive arachnoiditis on the dorsal surface at the C2, 3, 4 level and an extradural cyst communicating with the subarachnoid space through a dural defect on the left side of the C2 level. By excision of the extradural cyst and placement of a syringo-cisternal shunt, the syringomyelia shrank and the extradural cyst disappeared. It can be speculated that the pathogenesis of syringomyelia in this case was associated with ischemic change of the spinal cord due to compression and constriction caused by the extradural cyst and adhesive arachnoiditis.

Arachnoiditis↗
PubMed1987

[An autopsy case of syringomyelia associated with Chiari malformation and basilar impression].

The authors report one autopsy case of syringomyelia associated with Chiari malformation and basilar impression. The pathogenesis of syringomyelia in our case is discussed. This 37-year-old man complained of progressive difficulty in swallowing and walking for two years. He had noticed dysarthria for six months before admission. (Examination) Neurological examination showed dysarthria, down beat nystagmus, disturbance of IXth nerve, Xth nerve and XIth nerve, and cerebellar ataxia. Deep tendon reflexes were hyperactive in the upper and lower extremities. Babinski's sign was positive bilaterally. Neuroradiological examination demonstrated basilar impression and Chiari malformation. (Operation) Suboccipital craniectomy and laminectomy of upper cervical vertebra were performed with dural plasty. Postoperatively he acquired some improvement, but soon after he was worse. He died of respiratory disturbance. (Postmortem examination) Though the central canal was obliterated at the C4 level, the syrinx extended from the C5 to Th7 level. From the C5 to C8, the syrinx was present in the areas of central gray matter, extending into the left dorsal horn, where it communicated with subarachnoid space. Furthermore, the abnormal vessels were noticeable around the syrinx. At the Th2 level, they were also shown in central grey matter where no syrinx existed. (Conclusion) The etiology of syringomyelia in our case was not explained by Gardner's hydrodynamic theory. We suggested that intramedullary abnormal vessels played an important part for the formation of the syringomyelia.

Adult↗
PubMed1995

[Syringomyelia, a neglected differential diagnosis in multiple sclerosis. 6 cases from a specialty clinic for multiple sclerosis].

During the last 5 years 11 patients with syringomyelia have been found among 4348 patients (0.25%) entering our hospital, which specializes in multiple sclerosis. Six of these 11 patients had been diagnosed earlier as suffering from multiple sclerosis, some of them after a protracted course of neurological illness. In all 6 patients examination of the cerebrospinal fluid was normal, and visual-evoked potentials (VEP) were normal in all but one case, which is described in detail as case 2 in this report. Magnetic resonance imaging (MRI) showed a Chiari malformation in 3 of 6 syringomyelia patients, who came to us under the diagnosis of multiple sclerosis. MRI also showed subcortical white matter lesions in 5 of 6 patients with syringomyelia. In summary, the diagnosis of multiple sclerosis should be reexamined when one of the following signs is present: (1) demonstration of Chiari malformation; (2) cerebrospinal fluid is normal; (3) visual-evoked potentials are normal. These signs may suggest syringomyelia even after years of primary progressive or relapsing remitting development of multiple neurological deficits and MRI visible white matter abnormalities.

Adult↗
PubMedDate not supplied

Involvement of the autonomic nervous system in patients with syringomyelia--a study with the sympathetic skin response.

In order to determine the function of the autonomic nervous system in syringomyelia, the sympathetic skin response (SSR) was performed in 13 patients with syringomyelia and 20 healthy controls. SSR was recorded from both palms and soles. In patients with syringomyelia, we found absent responses, prolonged latencies and reduced amplitudes. SSRs could be recorded in 15 out of the examined 26 upper extremities. The latencies were prolonged in 12 of these cases. In the lower limbs, 11 SSRs could be obtained. In 4 of these cases the latencies were prolonged. The SSR latencies recorded from the palms and soles were both significantly prolonged (p < 0.05) and the amplitudes were reduced (p < 0.05) as compared to normal persons. Our data strongly suggest involvement of the autonomic nervous system in syringomyelia as assessed by the SSR response (in upper and lower extremities). In our patients, the extent of autonomic dysfunction was not related to the stage or the duration of disease.

Adult↗